IPAH CTD PoPH CTEPH LHD
Subjects n 15 8 18 14 19
Age (yrs) 48.3 ± 17.4 53.3 ± 20.1 48.9 ±8.3 68.711.2 67.8 ± 9.3
Gender (M/F) 2/13 0/9 11/7 6/8 5/14
IMC (Kg/m2) 25.8 ± 4,7 22.8 ± 2.5 30.3 ± 5.1 28.5 ± 5.2 30.4 ± 4.6
FVC () 91.9 ± 18.1 76.3 ± 32.7 104.2 ± 13.3 92.1 ± 21.4 87.1 ± 22
FEV1() 83.8 ± 23.7 73.9 ± 24.9 92.58 ± 13.6 83.4 ±26.2 80.5 ± 23.5
DLCOc/VA 73.6 ± 15.1 66.4 ± 13.3 65.7 ±15.7 85.9 ±28.5 89.1 ± 16.6
WHO FC ()          
I-II 26.7 37.5 61.1 21.4 31.3
III-IV 73.3 62.5 38.9 78.5 68.7
6MWT, m 401 ± 141.6 316.7 ± 81.1 462.4 ±110.2 302.8 ± 128.4 330.2±93
proBNP 2401.1 ± 3025.4 1246.1 ± 1450.5 695.1 ± 1216.9 2209.4 ± 3088.0 2523.1 ± 1777.8
EchocardiographysPAP   81.6 ± 82.7   67.6 ± 33.8   75.2 ± 25.2   70.7 ± 14   66.3 ± 15.5
Haemodynamics  
sPAP (mmHg) 71±22.6 61±21.7 70.7±21.7 69.5±19.6 59.7±16.2
dPAP (mmHg) 34,7±12.5 29.3±8.5 30.6±10.17 25.9±8.4 23.5±4.2
mPAP (mmHg) 47.6 ± 15.2 40.8 ± 14.5 46.7± 14.5 43.5 ±12.7 39 ± 9.4
PCWP, mmHg 10.4±3.8 12.4±3.2 19.7±6.7 14.5±7.3 21.2±8.4
TPG, mmHg 30.3±14.2 28.6±13.2 26.1±14.9 23.6±11.5 17.2±6.9
DPD, mmHg 19.2±10.8 14±8.4 10.8±11.1 12.4±9.2 4,7±4.9
CO (l.min-1) 4.8 ±1.8 3.1 ± 2.5 5.8 ± 2.4 4.1±1.5 4.6 ± 1.9
CI (l. min-1.m2) 2.6 ± 1.2 1.9 ±1.9 3.1 ± 1.3 2.2 ± 0.8 2.5 ± 1.1
PVR (din.seg.cm-5) 656.3 ± 416.3 418.151.5 ± 151.1 425.1 ±323.1 515.4±3.1 287.6 ±141.6
Values are shown as the mean ± SD. Abbreviations: IPAH (idiopathic PAH), CTD (connective tissue disease), PoPH (portopulmonary hypertension), LHD (left heart disease), WHO (World Health Organization), FC (functional class), 6MWT (6-minute walking test), sPAP (systolic pulmonary artery pressure), dPAP (diastolic pulmonary artery pressure), mPAP (mean pulmonary artery pressure), PCWP, mmHg (pulmonary capillary wedge pressure), TGP (transpulmonary gradient), DPD (diastolic pressure difference), CO (cardiac output), CI (cardiac index), PVR (pulmonary vascular resistance).
Table 2: Clinical and haemodynamic data at diagnosis for each PAH subgroup and CTEPH.